Common Atrioventricular Valve Research Articles

Mid-term outcomes of atrioventricular valve repair in functional single ventricle patients

BackgroundSurgical treatment of functional single ventricle combined with atrioventricular valve regurgitation remains a clinical challenge. The outcomes of atrioventricular valve repair in patients with single ventricle are limited.MethodsA retrospective study was conducted of all 28 patients with functional single ventricle treated with single-ventricle palliation who underwent atrioventricular valve operation at the First Hospital of Tsinghua University between April 2007 and October 2022.ResultsIn our cohort, the female/male ratio was 7:21, with an average age of 8.7 ± 6.0 (0.75–26) years. Half of patients (50%) were right-ventricle type for single-ventricle morphology. 18 patients (64.3%) were with a common atrioventricular valve. Twenty-three patients (82.1%) were combined with heterotaxy syndrome. Pre-operatively, twenty-four patients (85.7%) were diagnosed with severe atrioventricular valve regurgitation. AVV was repaired at the Glenn (n = 16, 57.1%), Glenn-Fontan (n = 2, 7.1%) and Fontan (n = 10, 35.7%) stage, respectively. Valve plastic techniques included valve annulus/commissure constriction (n = 24), clefts repair (9 cases), edge-to-edge suturing (13 cases) and common atrioventricular valve separation (4 cases). The early mortality was 3.6% (1/28). All survival patients were observed with improved regurgitation situations. Twenty-two patients (78.5%) were observed with no more than mild regurgitation postoperatively. The mean follow-up time was 5.4 ± 2.9 years (range, 3.08–11.83 years), with late mortality of 11.1% (3/27). All these three cases were observed with a severe regurgitation by echocardiogram in the last follow-up. Besides, reoperation rate of this cohort was 3.6% (1/28).ConclusionsAVV repair could significantly improve AVV function in SV patients combined with severe AVVR, with satisfactory mid-term results. Part of the cohort showed poor prognosis due to repeated AVVR. Regular follow-up by echocardiogram is critically important for these patients.

Surgical Outcomes of Patients With Heterotaxy Syndrome - The Japanese Congenital Cardiovascular Surgery Database.

Cardiac malformations are a major component of heterotaxy syndrome and result in significant mortality and morbidity. This multicenter nationwide Japanese study evaluated mortality and morbidity after initial surgical palliation for patients with heterotaxy syndrome and determined predictors for mortality and morbidity among patients enrolled in the study. The Japanese Congenital Cardiovascular Surgery Database (JCCVSD) collects clinical data from 119 domestic institutions specializing in congenital heart disease, covering almost all major congenital heart surgery programs in Japan. Clinical data on preoperative, operative, and postoperative characteristics and survival data within 30 and 90 days were available from the JCCVSD database. Of the 561 patients with heterotaxy syndrome who underwent any of 8 specific initial cardiovascular surgeries, 45 (8.2%) and 75 (13.4%) had died at 30 and 90 days, respectively. Preoperative emergency transport, type of heterotaxy syndrome, low hospital volume, the repair of total anomalous pulmonary vein connection, and the repair of a common atrioventricular valve were identified as significant predictive factors for operative mortality. Improvements in some medical circumstances, such as fetal diagnosis and the patient transport system, will be needed to improve outcomes for severely ill patients with heterotaxy syndrome. This study describes early outcomes for the largest number of main cardiovascular surgeries to date in infants with heterotaxy syndrome.

Optimal timing of bridging annuloplasty and patch augmentation for heterotaxy syndrome associated with functional single ventricles.

Although the challenging prognosis of functional single ventricles with common atrioventricular valves due to complex morphology and uncontrollable regurgitation by valvuloplasty has been highlighted, reports on when and how these extremely complicated atrioventricular valves should be repaired are few. This study investigated the timing and risk factors for valve intervention in these patients. Between April 2006 and March 2023, 40 patients with heterotaxy syndrome associated with functional single ventricles underwent surgery. Valve intervention was performed in 14 of the 40 patients with moderate or severe atrioventricular valve regurgitation. The timing of the first valve intervention varied, with four, five, three, and two patients undergoing valve intervention before the cavopulmonary shunt, simultaneously with the cavopulmonary shunt, before total cavopulmonary connection, and simultaneously with total cavopulmonary connection, respectively. Mechanical valve replacements were performed in three patients. Among the 14 patients undergoing valve intervention, four died. Three of the four patients underwent valvuloplasty before the cavopulmonary shunt, including two who could undergo the cavopulmonary shunt but died after the procedure. Eight of the fourteen patients completed total cavopulmonary connection. The cumulative survival rate was not significantly different between the 14 patients who underwent and 26 who did not undergo intervention (hazard ratio, 2.52; 95% confidence interval, 0.56-11.24; P = 0.23). Our surgical strategies provide a chance for the next staged repair of common atrioventricular valves in patients with both heterotaxy and valvular regurgitation. Including patch augmentation, advanced valve intervention is possible at or after the cavopulmonary shunt.

Clinical implications of dextrocardia based on four visceroatrial situs studies

BackgroundPrevious studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. MethodsWe retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs. ResultsDextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage. ConclusionOur study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.

An Unusual Presentation of Partial Atrioventricular Canal Defect in an Elderly Woman

A partial atrioventricular canal defect (PAVCD) is a form of endocardial cushion defect. It’s a rare congenital cardiac malformation, few reported patients have survived into the seventh decade of life. A 71-year-old female with a history of diabetes and hypertension was admitted for heart failure (HF) symptoms. Transthoracic echocardiography revealed a left to right shunt in the basal portion of the interatrial septum, 19mm ostium primum atrial septal defect (ASD), a mitral valve cleft, and severe mitral valve regurgitation. PAVCD consists of an ostium primum ASD and the dividing of the common atrioventricular (AV) valve into 2 separate AV orifices, resulting in a so-called cleft in the mitral valve. The ventricular septum is intact. Echocardiography is the key tool for diagnosis and anatomic classification. Surgical repair is recommended in adults with primum ASDs causing impaired functional capacity, right atrial or right ventricular enlargement, and left-to-right shunt without cyanosis, as long as systolic pulmonary artery pressures are lower than 50% of systemic pressures. Reconstructive surgery includes, in addition to the closure of the interatrial septum, a suturing of the mitral cleft. The estimated mean age at death of the patients with ostium primum ASD is about 40 years. The objective of this clinical case is to highlight the good clinical tolerance of atrial shunts and the anatomical particularities in the PAVCD.

Molecular Pathways and Animal Models of Atrioventricular Septal Defect.

The development of a fully functional four-chambered heart is critically dependent on the correct formation of the structures that separate the atrial and ventricular chambers. Perturbation of this process typically results in defects that allow mixing of oxygenated and deoxygenated blood. Atrioventricular septal defects (AVSD) form a class of congenital heart malformations that are characterized by the presence of a primary atrial septal defect (pASD), a common atrioventricular valve (cAVV), and frequently also a ventricular septal defect (VSD). While AVSD were historically considered to result from failure of the endocardial atrioventricular cushions to properly develop and fuse, more recent studies have determined that inhibition of the development of other components of the atrioventricular mesenchymal complex can lead to AVSDs as well. The role of the dorsal mesenchymal protrusion (DMP) in AVSD pathogenesis has been well-documented in studies using animal models for AVSDs, and in addition, preliminary data suggest that the mesenchymal cap situated on the leading edge of the primary atrial septum may be involved in certain situations as well. In this chapter, we review what is currently known about the molecular mechanisms and animal models that are associated with the pathogenesis of AVSD.

The impact of surgical repair on left ventricular outflow tract in atrioventricular septal defect with common atrioventricular valve orifice

ObjectiveAlthough a narrow left ventricular outflow tract in atrioventricular septal defect is related to its intrinsic morphology, the contribution from the repair technique remains to be quantified. MethodsA total of 108 patients with an atrioventricular septal defect with a common atrioventricular valve orifice were divided into 2 groups: 2-patch (N = 67) and modified 1-patch (N = 41) repair. The left ventricular outflow tract morphometric was analyzed by quantifying the degree of disproportion between subaortic and aortic annular dimensions (disproportionate morphometrics ratio was defined as ≤ 0.9). Z-scores (median, interquartile range) were further analyzed in a subset of 80 patients with immediate preoperative and postoperative echocardiography. A total of 44 subjects with ventricular septal defects served as controls. ResultsBefore repair, 13 patients (12%) with an atrioventricular septal defect had disproportionate morphometrics (vs 6 [14%] ventricular septal defect P = .79), but the subaortic Z-score (−0.53, −1.07 to 0.06) was lower than the ventricular septal defect (0.07, −0.57 to 1.17; P < .001). After repair, both 2-patch (8 [12%] preoperatively vs 25 [37%] postoperatively; P = .001) and modified 1-patch (5 [12%] vs 21 [51%], P < .001) procedures showed a greater degree of disproportionate morphometrics. Both 2-patch (postoperatively −0.73, −1.56 to 0.08 vs preoperatively −0.43, −0.98 to 0.28; P = .011) and modified 1-patch (−1.42, −2.63 to –0.78 vs −0.70, −1.18 to −0.25; P = .001) procedures also demonstrated lower subaortic Z-scores postrepair. The postrepair subaortic Z-scores were lower in the modified 1-patch group (–1.42 [−2.63 to −0.78]) compared with the 2-patch group (–0.73 [−1.56 to 0.08]; P = .004). Low postrepair subaortic Z-scores (<−2) were observed in 12 patients (41%) in the modified 1-patch group and 6 patients (12%) in the 2-patch group (P = .004). ConclusionsSurgical correction resulted in greater disproportionate morphometrics seen immediately postrepair. The impact on the left ventricular outflow tract was observed in all repair techniques, with a greater burden seen after modified 1-patch repair. Video AbstractThis morphometric study in AVSD with common atrio-ventricular valve orifice confirmed further derangements of LV outflow tract morphometrics immediately after surgical repair.

The Fontan procedures – early outcomes and predictive factors

Objectives: the aim of this study was to describe the early outcomes after the extracardiac conduit Fontan procedure and evaluate predictive factors related to these results.&#x0D; Methods: a cross – sectional study. We retrospectively reviewed the perioperative medical records of 71 patients with Fontan surgery at Hanoi Heart Hospital between January 2017 and December 2020. Data processing using SPSS 22 software&#x0D; Results: the early mortality and early failure rates with Fontan circulation were 4.2%. High postoperative pulmonary artery pressure was a risk factor for early mortality and early Fontan failure. The mean duration of mechanical ventilation was 62.86±108.17 hours; female, short height, and common atrioventricular valve or double­inlet atrioventricular connection increased the risk of prolonged mechanical ventilation. The mean intensive care unit (ICU) length of stay was 6.46±6.32 days, female, short height, and moderate to severe postoperative atrioventricular valve regurgitation increased the risk of prolonged duration in ICU. The mean time of pleural drainage was 16.96±10.23 days, low weight was a risk factor for prolonged pleural drainage. The mean postoperative hospital stay was 23.89±11.83 days, age at the Fontan surgery, preoperative oxygen saturation, concomitant atrioventricular valve repair, and postoperative atrioventricular valve regurgitation were factors associated with prolonged postoperative hospital stay.&#x0D; Conclusion: the early outcomes after Fontan surgery have been consistent with our condition. There have been many risk factors that influenced early results after surgery, and identifying those factors may help with our treatment.

Kawashima Procedure: The Impact of Age

BackgroundThe results of the Kawashima operation are incompletely defined. Furthermore, optimal timing of Kawashima operation, an important consideration when managing desaturated young infants awaiting surgery, remains unclear. We reviewed our outcomes of Kawashima operation, with a focus on the impact of age. MethodsWe conducted a retrospective review of patients who underwent Kawashima operation from 1990 to 2020. ResultsThirty patients underwent Kawashima operation at a median age of 11.7 months (interquartile range, 4.4-27.4). Left isomerism was present in 27 patients (90%). There were no early deaths. There were 2 patients (6.7%) who had Kawashima takedown, both attributed to hypoxia. Fontan completion was achieved in 25 patients (83%). Overall freedom from death and transplantation at 20 years was 67% (95% CI, 32%-87%). Freedom from death and transplantation at 10 years was 100% for children 3 to 6 months of age at the time of Kawashima, compared with 86.4% for older children (P = 1.0). However, children aged 3 to 6 months had longer ventilation time (P = .01), intensive care stays (P = .03), and hospital stays (P = .05). Freedom from death or transplantation at 20 years among those who had concomitant common atrioventricular valve repair was 33%, vs 79% for those who did not undergo common atrioventricular valve repair (P = .02). ConclusionsKawashima operation can be performed with low operative risk and acceptable long-term outcomes. Performing Kawashima operation on a patient aged less than 6 months does not affect survival, but is associated with increased morbidity. Need for common atrioventricular valve operation carries significant risk of mortality, and more effective techniques for atrioventricular valve repair are required.

Pentalogy of Cantrell.

Pentalogy of Cantrell.

Prenatal ultrasound diagnostics of atrioventricular communication in combination with the tetralogy of Fallot in a fetus with Down's syndrome phenotype

This article presents a description of our own case of ultrasound prenatal diagnostics of complex combined heart disease (complete atrioventricular communication in combination with tetralogy of Fallot) at 19–20 weeks of gestation with ultrasound markers of Down's syndrome in fetus. Pregnancy termination was performed at 21 weeks. Prenatal diagnosis was fully confirmed at the fetus autopsy. This publication discusses the frequency, mechanism of occurrence of such a combination of congenital heart defects, prenatal management, methods, timing and efficiency of surgical correction after birth. Complete atrioventricular communication occurs in approximately 1.7–2% patients with tetralogy of Fallot, and tetralogy of Fallot occurs in 5–10% of patients with atrioventricular communication, most often in patients with Down's syndrome. It is believed that tetralogy of Fallot and a complete defect of the atrioventricular septum develop as a result of different mechanisms, but their joint occurrence is recognized as an association. The prevalence of such a combination in Down syndrome indicates the general foundations of its development.Sonographic diagnosis of atrioventricular communication and tetralogy of Fallot is usually not difficult for prenatal screening. Main diagnostic criteria for complete atrioventricular communication at routine ultrasound screening and targeted fetal echocardiography are an abnormal 4-chamber image of the heart in the form of a common atrioventricular junction and a common five-leafed atrioventricular valve. Typical prenatal sonographic signs of the tetralogy of Fallot are a high defect of the interventricular septum, expansion of the aortic root, displacement of the aorta forward and to the right, narrowing of the pulmonary artery at the level of the valve ring from minor to complete atresia, it is also possible reduction the size of the right ventricle and increasing the size of the left ventricle.The combination of atrioventricular communication with the tetralogy of Fallot refers to severe congenital heart defects requiring surgical correction.

Atrioventricular septal defect in Fontan circulation: Right ventricular dominance, not valve surgery, adversely affects survival

ObjectiveThe effect of ventricular dominance and previous atrioventricular valve (AVV) surgery on patient outcomes after Fontan operation remains unclear. We sought to determine the effect of ventricular dominance and previous AVV surgery on transplantation-free survival and long-term AVV competency in patients with atrioventricular septal defect (AVSD) and Fontan circulation. MethodsWe conducted a retrospective study of 1703 patients in the Australia and New Zealand Fontan Registry, who survived Fontan operation between 1987 and 2021. ResultsOf 174 patients with AVSD, 60% (105/174) had right ventricular (RV) dominance and 40% (69/174) had left ventricular (LV) dominance. The cumulative incidence of moderate or greater AVV regurgitation at 25 years after Fontan operation in patients with LV dominance was 56% (95% CI, 35%-72%), compared with 54% (95% CI, 40%-67%) in patients with RV dominance (P = .6). Nonetheless, transplantation-free survival at 25 years in patients with LV dominance was 94% (95% CI, 86%-100%), compared with 67% (95% CI, 52%-87%) in patients with RV dominance (hazard ratio, 5.9; 95% CI, 1.4-25.4; P < .01). Of note, transplantation-free survival was not different in patients who underwent AVV surgery before or at Fontan completion compared with those who did not (15 years: 81% [95% CI, 62%-100%] vs 88% [95% CI, 81%-95%]; P = .3). ConclusionsIn patients with AVSD and Fontan circulation the rate of moderate or greater common AVV regurgitation is similar in those with LV and RV dominance. RV dominance, rather than previous AVV surgery, is a risk factor for death or transplantation.

Use of Isolated Right Ventricular Assist Device for Refractory Graft Failure Following a Pediatric Orthotopic Heart Transplantation

<h3>Introduction</h3> Mechanical circulatory support for isolated right ventricular (RV) failure following heart transplantation (HT) is well described in the adult literature. However, there are no similar pediatric reports . We report a case of successful use of paracorporeal CentriMag ventricular assist device as a bridge to recovery for acute RV failure following HT. <h3>Case Report</h3> This is a 12-month-old with complex congenital heart disease; single ventricle (SV), common AV valve, pulmonary atresia, heterotaxy, with dextrocardia, situs inversus and interrupted inferior vena cava with azygous continuation to a left superior vena cava. Stage 1 palliation with a ductal stent was performed in the neonatal period. She was deemed not a suitable candidate for SV palliation due to poor ventricular function as such was listed for HT. She underwent a complex HT operation including hilum-to-hilum pulmonary artery reconstruction. Intraoperative echocardiogram showed reasonable left ventricular function but poor RV function with high central venous pressure(CVP). Despite increasing inotropic support she remained hypotensive. A decision was made to place the patient on RVAD using a CentriMag device, with configuration from the right atrium to the main pulmonary artery. The patient hemodynamics improved immediately and decreased CVP. Patient was supported on RVAD for 72h before explantation. She was extubated on post op day 4 and discharged home after two weeks. At six months post HT she remains with normal graft function. <h3>Summary</h3> Acute RV dysfunction after HT is attributed to either an increase in pulmonary vascular resistance or loss of contractility in the donor heart. In this patient, a combination of pulmonary hypertension, diffuse hypoplasia of the pulmonary arteries and long donor ischemic time contributed to RV dysfunction. The present case is unique in that this patient was successfully supported with isolated RVAD, avoiding VA ECMO which is traditionally the modality of choice in such a case scenario. To the best of our knowledge, this is first case report of an infant supported on RVAD following HT. Consideration of RVAD to manage refractory primary graft failure related to RV dysfunction following HT should be considered especially in the absence of need for pulmonary or left ventricular support.

A case of esophageal atresia complicated by a right-sided aortic arch with right ductus arteriosus and inferior vena cava interruption with hemiazygos continuation

During the repair of esophageal atresia with tracheoesophageal fistula (EA/TEF), cardiovascular malformations occasionally create a technical challenge. We report a novel case of a 4-day-old girl with EA/TEF and multiple cardiovascular malformations, including right-sided aortic arch (RAA), right ductus arteriosus (RDA), single ventricle with single atrium, common atrioventricular valve, pulmonary atresia, bilateral superior vena cava, and interruption of the inferior vena cava (IVC) with hemiazygos continuation. In this case, a right-sided approach would require the mobilization of the RAA and RDA, which were supplying the pulmonary blood flow due to pulmonary atresia. Alternatively, the left-sided approach would require the mobilization of the hemiazygos vein, which was essential for venous return from the lower body due to IVC interruption. We performed the less intrusive left-sided approach, and the postoperative course was uneventful. Right-sided EA/TEF repair should be avoided because RDA spasm or injury caused by RAA mobilization would be fatal. In cases of interrupted IVC with azygos or hemiazygos vein continuation, care must be taken not to ligate these vessels or block the venous return. Preoperative evaluation is important to prevent complications in such complicated cases. If sufficient information is not available, the left-sided approach may be preferred. • In case of RAA with RDA, the EA/TEF repair should be approached from the left side. •Detection of cardiovascular malformations is necessary in case of EA/TEF with RAA. •This is the first case report of EA/TEF with RAA that clearly records RDA.

Repair of Common Atrioventricular Valve in Univentricular Circulation Using Adjustable Annular Bridging Technique

The main reason for common atrioventricular valve regurgitation is the enlargement of the valve annulus contralaterally to the aorta and pulmonary artery. Edge-to-edge repair can be used to address the regurgitation; however, there might be potential disadvantage that a fixed bridging leaflet could have a restrictive motion, leading to recurrence of atrioventricular valve regurgitation. To avoid this issue and address the anteroposterior dilatation of the annulus, we have developed an adjustable annular bridging technique. The main reason for common atrioventricular valve regurgitation is the enlargement of the valve annulus contralaterally to the aorta and pulmonary artery. Edge-to-edge repair can be used to address the regurgitation; however, there might be potential disadvantage that a fixed bridging leaflet could have a restrictive motion, leading to recurrence of atrioventricular valve regurgitation. To avoid this issue and address the anteroposterior dilatation of the annulus, we have developed an adjustable annular bridging technique. Commentary: The Adjustable Bridge – Definitely a Useful Technique; HoweverOperative Techniques in Thoracic and Cardiovascular SurgeryVol. 27Issue 3PreviewI am grateful to the authors and commend them for sharing their technique of adjustable annular bridge for patients with single-ventricle (SV) hearts and atrioventricular valve regurgitation (AVVR).1 They have provided clear technical detail and excellent illustrations and video. No one questions the significance of AVVR in these patients, its significant impact on prognosis, and the difficulty in achieving durable, excellent valve competence. Likewise, we acknowledge the numerous technical repair tools at our disposal and that frequently we use most, if not all, of them in any given case (side to side, cleft or scallop closure, various degrees of annuloplasty, etc.). Full-Text PDF Commentary: Atrioventricular Valve Repair in Univentricular Hearts: No Longer a Bridge Too Far?Operative Techniques in Thoracic and Cardiovascular SurgeryVol. 27Issue 3PreviewIt is increasingly apparent that atrioventricular valve (AVV) failure is a major cause of mortality in patients with a Fontan circulation.1 Furthermore, patients with a common AVV are at the highest risk of AVV failure among those with a Fontan circulation.1-4 Atrioventricular valve repair is notoriously difficult in the setting of a common AVV, and failed repair is associated with very high mortality.3 One of the major contributing factors to AVV failure in these patients is the attenuated fibrous skeleton of the heart, which increases the risk of annular dilatation. Full-Text PDF

Atrioventricular Valve Repair in Patients with Univentricular Circulation

Atrioventricular valve (AVV) disease remains a challenge in the management of patients with congenital heart disease, particularly those with single ventricle palliation. Within this group, atrioventricular valve (AVV) disease remains the Achilles heel of long-term successful palliation. In recent years we have learned how AVV pathology is detrimental to late survival, as well as how challenging it can be to obtain a competent and durable repair. In this expert review we outline strategies for AVV repair depending on AVV morphology: mitral, tricuspid, or common atrioventricular valve. Given the broad scope of AVV pathology, a large number of tools are needed in a congenital cardiac surgeon's armamentarium to successfully treat these patients. Many of these techniques have been successfully employed in patients with biventricular physiology, but can have applicability to those with single ventricle palliation.

A surgical approach of an unusual variant of complete atrioventricular defect; A case report

BackgroundComplete atrioventricular canal is a congenital heart defect that is characterized by an atrial septal defect, ventricular septal defect, and a common atrioventricular valve. Standard surgical techniques for repairing complete atrioventricular canal defect mainly includes repairing the defect with a single patch, a double patch, or with the modified single patch technique.Case presentationThis paper presents a novel surgical repair technique of an unusual anatomical presentation for a complete atrioventricular canal defect in a patient with Down syndrome.ConclusionsUnusual anatomical variant for congenital heart defects occurs frequently, which gives surgeons real opportunities to innovate surgical approaches. This patient was an example of an unusual anatomical presentation for complete atrioventricular canal, and the surgical technique used for this patient was novel. Follow up for these patients is mandatory for long term results.

Surgery for Functional Single Ventricle Associated with Heterotaxy Syndrome: Common Atrioventricular Valve Repair

Surgery for Functional Single Ventricle Associated with Heterotaxy Syndrome: Common Atrioventricular Valve Repair

Further clarification on the variants of double-outlet right atrium.

Uniatrial but biventricular atrioventricular connection is a rare congenital cardiac abnormality where the left atria-ventricular connection is absent and the right atrio-ventricular connection straddles the crest of the muscular ventricular septum. This anomaly has been referred to as double outlet right atrium and the atrio-ventricular valve as a common atrioventricular valve in the past. In the absence of a primary atrial septal defect, the atrio-ventricular junction is not a common junction and the valve cannot hence be described as a common trio-ventricular valve. We demonstrate this paradox by an illustrative case and contrast this with two other cases where a common atrio-ventricular valve was predominantly committed to the right atrium.

The effect of Surgical Technique, Age and Trisomy 21 on early outcome of Surgical Management of Complete Atrioventricular Canal Defect

Abstract Background The optimal timing, surgical technique and the influence of Down syndrome on outcome of surgical repair of Complete Atrio-Ventricular Canal Defect (CAVC) remains uncertain. We reviewed our experience in repair of CAVC to identify the influence of these factors on operative outcome. Methods A prospective study included 70 patients who underwent repair of CAVC at Ain Shams university hospitals, academy of cardiothoracic surgery during the period from July 2016 to October 2019. Age at surgery (less than 6 months old versus later), surgical technique used [(modified single patch versus double patch technique), (use of posterior annuloplasty for repair of left AV valve or not)] and association of Down syndrome were evaluated for their impact on the outcome of surgical repair using multivariate analysis. Patients were followed up for 6 months; primary end point was mortality and secondary end point was reappearance of LAVV regurgitation. Results No significant difference between patients operated on, at the first 6 months of age versus later, regarding mortality or LAVV regurgitation. Down patients showed significant difference in the occurrence of postoperative compared to non-Down patients (LAVVR grade 2 + = 8.9% vs 24%, P value =0.005) respectively. Surgical repair by Modified single patch technique showed significant reduction in cross clamp time (mean = 47.6 ± 9.227 min vs 73.55 ± 21.087 min, P value 0.00), shorter bypass time (mean = 71.13 ± 13.507 min vs 99.19 ± 27.092 min, P value =0.00) and shorter duration of ICU stay (mean =3.2 ± 1.657 days vs 5.3 ± 2.761 days, P value=0.01) as compared to double patch technique. Posterior annuloplasty used for repair of LAVV compared to closure of cleft only resulted in significant reduction in the occurrence of post-operative valve regurgitation during the early period (LAVVR 2+ 43% vs 7%, P value=0.03) and at 6 months of follow up. (LAVVR 2+ 35.4% vs 0%, P value=0.01) respectively. Conclusion early intervention, in the first 6 months in patients with CAVC by surgical repair gives comparable acceptable results to later repair, Down syndrome was not found to be a risk factor for early intervention. Modified single patch and double patch techniques for repair, can be used both with comparable results even in large VSD component (8mm and larger), finally, repair of common AV valve by cleft closure with posterior LAV annulplasty showed better results with significant decrease in postoperative LAV regurgitation and early mortality in comparison to closure of cleft only.